Wegener's granulomatosis in a pediatric patient.

Wegener's Granulomatosis (WG) is characterized by the clinical triad of upper and lower respiratory disease, and renal insufficiency. Because WG masquerades as a syndrome, its diagnosis is often delayed. Astute clinicians should include WG in their differential diagnosis when a patient presents with these classic findings. The diagnosis is a clinicopathologic one made by biopsy of appropriate tissue showing necrotizing granulomatous vasculitis. Lung tissue obtained by open thoracotomy has the highest diagnostic yield. At one time a universally fatal illness, WG is now treatable with cyclophosphamide and prednisone therapy with remission in most cases. The authors present a case report of a pediatric patient who was eventually diagnosed with WG. The case serves as a reminder to all clinicians to keep this entity as a part of their diagnostic armamentarium.